(Last Updated On: October 30, 2018)

Authors: Ma. Corazon A. Iniego, MD, Alexis Paula Ibanez, MD, Elizabeth P. Prieto, MD, FPDS

Abstract

Introduction: Paraneoplastic pemphigus is an autoimmune blistering disease commonly associated with lymphoproliferative disorder and rarely with a carcinoma. It is characterized by the presence of intractable, painful stomatitis and polymorphous skin eruption. There is no clear racial and gender predilection. IgG and complement deposition in the epidermal intercellular spaces and granular or linear complement deposition along the epidermal basement membrane zone can be appreciated on direct immunofluorescence.

Case Summary: A 61-year old female came to several ENT specialists and dentists due to four-month history of intractable, painful stomatitis with crusting and erosion extending to the vermilion borders. She was initially managed as oral candidiasis and was given several doses of itraconazole and unrecalled mouthwash without resolution of symptoms. Chest Xray revealed right pulmonary mass and chest CT scan showed a 12.3x7x7.9 cm heterogeneously enhancing soft tissue mass in the anterior segment of the right upper lobe. CT-guided biopsy showed scant clusters of small, mostly round to spindled cells, with scant cytoplasm. She developed multiple, well defined, round- oval shaped, clear fluid-filled, tense and flaccid vesicles and bullae over the trunk, abdomen, back, upper extremities, gluteal and lower extremities some arranged annularly. Skin punch biopsy was consistent with intraepidermal blistering disease. Direct immunofluorescence was positive for intercellular IgG and C3 in the lower half of the epidermis.

Conclusion: Paraneoplastic pemphigus is a rare, life-threatening, autoimmune blistering disease, commonly associated with lymphoproliferative malignancy. The plakin protein family are the targeted antigens. Management usually involves treating the underlying malignancy and mortality rate is as high as 90%.

 

Citation

 

Keywords

Paraneoplastic pemphigus, immunodermatology

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